Pediatric cataract

Pediatric Cataract

1. PEDIATRIC CATARACT Presenter: Dr. Ajay Kumar Singh Moderator: Dr. Wangchuk Doma Venu Eye Institute & Research Centre, New Delhi

2. INTRODUCTION Pediatric cataract Congenital cataract (present at birth) Developmental cataract (develops soon after birth) Traumatic cataract

3. INTRODUCTION Incidence - 6/10,000 10% of childhood blindness Congenital or Acquired Visually significant or not Stable or Progressive Unilateral or Bilateral Partial or Complete Developmental opacity are usually partial & stationary

4. ANATOMY Lens - A transparent,biconvex,crystalline structure between iris & vitreous in a saucer shaped depression called patellar fossa . Components Anterior capsule Anterior capsular epithelium Cortex Nucleus Posterior capsule

5. CONGENITAL VERSUS DEVELOPMENTAL Congenital & developmental cataract not synonymous In widest sense, most forms of cataract (even senile cataract) may be considered as developmental, for human lens grows until late in life Criteria for congenital cataract present at birth situated within fetal nucleus diameter of opacity less than 5.75mm (diameter of newborn lens) If size of opacity more than 5.75mm,cataract must be postnatal

6. Genetic Mutation Autosomal Dominant Metabolic Galactosaemia Lowe Hypoparathyroidism Fabry Infective TORCH organisms Congenital rubella Chromosomal Trisomy 21 (Down) Trisomy 18 (Edward) Trisomy 13 ( Patau ) Skeletal Hallerman - Streiff -Francois syndrome Nance-Horan syndrome Ocular anomalies Aniridia Anterior segment dysgenesis syndrome Idiopathic in 50% Congenital cataracts: Bilateral

7. Congenital cataracts: Unilateral Sporadic no family history/systemic disease Cause identified in only 10% Ocular anomalies Persistent fetal vasculature (most common) Affected infants usually full term & healthy

8. MORPHOLOGY TYPES Punctate (blue dot) cataract Anterior Capsular cataract Anterior polar cataract Pyramidal Reduplicated Posterior polar cataract Embronal (nuclear) cataract Coronary cataract/ Fusiform cataract Zonular (Lamellar) cataract Total cataract Soft Membranous

9. MORPHOLOGICAL TYPES

10. PUNCTATE(BLUE DOT) CATARACT Most common type,universal Cataracta coerulea /blue dot cataract Multiple tiny blue spots scattered all over lens esp in cortex Variant – cataracta centralis pulverulenta dominantly inherited,non progressive central spheroidal /biconvex opacity consisting of powdery fine white dots within embronic /fetal nucleus Non progressive&visually insignificant

11. ZONULAR (LAMELLAR) CATARACT Accounts for 50% of visually significant cataract May be congenital or occurs at a later stage as development interfered at a later stage Zone around embryonic nucleus (usually in area of fetal nucleus) become opacified,extent depending on duration of inhibiting factor Opacity sharply demarcated . Area of lens within & around opaque zone is clear

12. Linear opacities like spokes of a wheel (called riders) may run outwards Usually bilateral,formed just before/shortly after birth Fill pupillary aperature when pupil undilated , thus affecting vision Often hereditary ( autosomal dominant) Associated with hypovitaminosis D (with evidence of ricket ) or hypocalcemia (with tetany or defective tooth enamel)&maternal malnutrition ZONULAR (LAMELLAR) CATARACT

13. CORONARY CATARACT Around puberty Situated in deeper layers of cortex &superficial layers of adolescent nucleus Corona or club shaped opacities near periphery of lens ,usually hidden by iris while axial region &extreme periphery remain clear Non progressive & does not interfere with vision

14. NUCLEAR CATARACT Associated with rubella Incidence more if infection contracted in 2 nd month Development of lens inhibited at very early stage Embyonal nuclear cataract Progressive ,becomes total cataract Associated microphthalmos , nystagmus,strabismus,glaucoma,iris hypoplasia & pigmentary retinopathy

15. ANTERIOR CAPSULAR CATARACT May be due to Delayed formation of anterior chamber (Developmental) Acquired (commonly)-follows contact of capsule with cornea, usually after perforation of ulcer in ophthalmia neonatorum Three types- Anterior polar (flat type) Anterior pyramidal Reduplicated

16. Flat type – white plaque forms in central pupillary area less than 3mm in diameter visually insignificant Pyramidal type – sometimes central plaque projects forwards into anterior chamber like a pyramid Frequently surrounded by area of cortical opacity may affect vision ANTERIOR CAPSULAR CATARACT

17. Subcapsular epithelium may grow in between capsule & cortical opacity Clear lens fibre subsequently growing from there lay down a transparent zone between two opacities Buried opacity is called imprint & two together constitute Reduplicated cataract Association – Persistant pupillary membrane Aniridia Peters anomaly Anterior lenticonus ANTERIOR CAPSULAR CATARACT

18. POSTERIOR CAPSULAR ( POLAR) CATARACT Associated with persistent hyaloid remnants( mittendorf dots),posterior lenticonus & persistent anterior fetal vasculature Common in minimal degree & visually insignificant With persistent hyloid artery , lens deeply invaded by fibrous tissue leading to total cataract

19. OTHER TYPES SUTURAL CATARACT Opacity follows anterior /posterior Y suture May occur in isolation or association with other opacity MEMBRANOUS CATARACT Rare Associated with Hallermann - Streiff -Francois syndrome occurs when lenticular material partially /completely reabsorbs leaving behind residual chalky –white lens matter sandwiched between anterior & posterior capsule

20. Symptoms of Developmental Cataract Informant – usually parents History of white spot in pupillary area Child is usually brought with history of diminution of vision / does not recognize objects and parents Unsteady eyes Deviation of eye Associated symptoms of systemic disease, if present

21. Signs Diminished vision (at times it is difficult to establish in very young children) Lenticular opacity Nystagmus Deviation of eye There may be other ocular and systemic abnormalities in cases of rubella nuclear cataract

22. Management of Developmental Cataract Investigations Detailed history Complete ocular examination- UCVA, BCVA, pupillary reaction intra-ocular tension, fundus examination B scan ultrasonography to exclude posterior segment abnormality like growth/ retinoblastoma A scan to determine axial length of the eye Retinoscopy , to deremine the refractive status cover test to exclude squint Early photographs to know onset of cataract

23. Laboratory investigations: (For bilateral cases) Blood Test Full blood count,Blood glucose(FBS/RBS) Serum calcium and phosphorus RBC transferase and Galactokinase levels TORCH test Hepatitis B virus Urine analysis: For reducing substance for galactosaemia For amino acids (to exclude Lowe syndrome in suspected cases) U/L Pediatric cases are mostly idiopathic. No need of lab investigation Management of Developmental Cataract

24. Cataract in childhood not only reduce vision but also interfere with normal visual development Timing of surgery ,surgical technique,choice of aphakic correction & amblyopia management are of utmost importance to achieve good &long standing results The inflammatory response to surgical results are more pronounced in children Post operative amblyopia management forms an integral part of visual rehabilitation in children Management of Developmental Cataract

25. ?????? To operate or not? If yes, when to operate? If no, how to manage? If operating, should one place the IOL? If not placing IOL, how to manage and follow the child?

26. Non-surgical treatment Used for partial cataracts Partial cataract less than 3 mm and pericentral cataracts respond Pupillary dilatation with 2.5% phenylephrine and part time occlusion of good eye Cyclopentolate can be added once or twice a day if required Prolonged cyclopegia (daily atropine) can induce amblyopia Pupillary dilatation is reserved for preverbal (1-6 years) with partial cataracts and borderline amblyopia If significant amblyopia persists, cataract extraction should be performed

27. WHEN TO OPERATE?? Bilateral dense cataracts : - require early surgery at 4-6 weeks of age to prevent development of stimulus deprivation amblyopia Bilateral partial cataracts -may not require surgery or require only at a later date

28. 3.Unilateral dense cataract -urgent surgery is advised within days -aggressive anti- amblyopia therapy should follow surgery -results are often poor -if detected after 16 weeks of age then surgery is inadvisable because amblyopia is refractory 4.Partial unilateral cataract may be treated non surgically

29. General techniques Deep general anaesthesia is required Pediatric cataracts are soft – lens material can be aspirated through incisions that are 1-1.5mm long at the limbus ; can be subjected to lensectomy through pars plana A larger wound is needed to introduce IOL Tunnel should be securely sutured to prevent dehiscence of wound with iris incarceration

30. Specific techniques There are two approaches Pars plana approach Limbal approach Pars plana approach is being abandoned gradually in favour of limbal approach as limbal approach allows better preservation of the capsular bag for in-the-bag IOL placement.

31. Pars plana approach Indications : neonates and infants <2years of age with B/L cataracts for whom IOL implantation is not intended immediately

32. ADVANTAGES Rapid and allows a permanently clear visual axis Less complicated post-operative course Iris and corneal endothelium suffer less iatrogenic damage Two eyes can be operated at the same time reducing the risk of relative amblyopia and second exposure to general anaesthesia DISADVANTAGES Incarceration of the vitreous in the scleral incisions Subsequent vitreal tractions and retinal breaks or detachments Insufficiently dilated pupil hinders lensectomy leading to too little capsular support for future IOL implantation

33. Limbal approach Most versatile technique Advantages: visualization of the instruments the ability to preserve the posterior capsule when desired, and the lack of disruption of the vitreous unless planned.

34. Aphakic correction in children Spectacles Contact lenses IOLs

35. Spectacles Satisfactory only in cases of B/L aphakia Most develop good visual acuity Cosmetically not acceptable Poor optical quality of high –plus lenses

36. Contact lenses Better optical correction than spectacles Dioptric power can be adjusted throughout the life Difficult to manage and costly Loss of lenses Frequent infections Poor follow up Thus most impractical

37. Intraocular lenses The IOL facilitates amblyopia management by providing a more permanent correction Implanting an IOL in a growing eye is not an ideal solution, but it is currently the most practical one. The aim in the IOL option is to correct most, but not all, of the aphakia the residual refractive error has to be corrected using spectacles, which can be adjusted throughout life. Posterior chamber IOL implantation is the safe method

38. Both the biometry and the age of the child determine the choice of the IOL dioptric power. Age <2 years- axial length and the keratometric (K) readings change rapidly Age 2-8 years- changes are slower and more moderate. Expected large Myopic Shift AIM FOR UNDERCORRECTION Selection of IOL

39. GUIDELINES FOR THE CHOICE OF INTRAOCULAR LENS DIOPTRIC POWER CHILDREN LESS THAN 2 YEARS OLD    •    Do biometry and undercorrect by 20%    •    Use axial length measurements only:        - 17mm, 25 D    -18 mm, 24 D    -19 mm, 23 D     -20mm, 21 D    -21 mm, 19 D CHILDREN BETWEEN 2 AND 8 YEARS OLD    •    Do biometry and undercorrect by 10%

40. IOL Implantation in children After the cataract has been aspirated, an elective posterior capsulectomy-anterior vitrectomy is performed. Sulcus implantation is easier and also allows an easier explantation - may be done in neonates and infants less than 1 year of age. But with the newer foldable IOLs, in the bag implantation is the preferred technique. An in-the-bag IOL is more difficult to explant, this option should be chosen for infants above 1 year of age because they are less likely to need an IOL exchange, provided they are undercorrected by 20%.

41. IOL Implantation in children In the bag technique requires both anterior and posterior capsulorrhexises The IOL haptics are placed in the bag fornices, while the optic is protruded through both capsulorrhexises to be captured beneath the posterior capsule remnants. Tassignon has developed a special IOL called bag-in-the-lens . The technique consists of creating an anterior and posterior capsulorrhexis . The specially designed IOL has, at its periphery, a groove that contains both anterior and posterior capsule rims

42. Complications Intraoperative complications -The anterior chamber tends to collapse - the iris can protrude through the surgical wounds - the pupil constricts on injury to the iris Immediate postoperative complications -anterior plastic uveitis - high intraocular pressure, -incarceration of iris tissue in the wound - endophthalmitis Late complications -dislocation of the IOL - chronic iritis - glaucoma -retinal detachment

43. Amblyopia management The unilateral cases are the most difficult to manage Amblyopia treatment starts soon after surgery, after clarification of the media. The initial treatment must be aggressive Full-time occlusion of the sound eye is carried out for a few days - 1 day per month of age Thereafter, occlusion is reduced to half the waking hours. Autorefractometers help to determine the residual refractive error Spectacles are prescribed from the age of 4 months onward.

44. A bifocal lens with an add of +3.00 is prescribed in the pseudophakic eye from the age of 3 years, when the child becomes verbal. Unilateral pseudophakes should continue with half-day patches until 4-5 years of age. Thereafter, the patch time can be reduced gradually, but should not be abandoned until 10–12 years of age. After that age, amblyopia management is practically superfluous. Cases of bilateral pseudophakia should be followed closely to detect and treat relative amblyopia.

45. Intraocular Lens Exchange and Alternative Options Exchange of IOLs should be considered when a great myopic shift has occurred When the pseudophakic eye becomes 7 D more myopic than the sound eye, the IOL should be exchanged Refractive surgery in children is not yet an acceptable option An alternative to IOL exchange is to implant an additional negative dioptric power IOL in posterior chamber to correct the myopia. This procedure is easily performed when the primary IOL was inserted in the bag.

46. PROGNOSIS Visual outcome depends on - Type of cataract - Timing of intervention - Quality of life - Amblyopia management Near normal vision can be achieved in U/L congenital cataract, provided amblyopia management is aggressive Binocularity is usually poor Aphakic & pseudophakic children should be followed throughout childhood &preferably throughout life

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